QOTW – ‘What non-pharmaceutical treatments are available and being supported by HAEA and the physicians?’

Nov 2, 2015

Dr. C:This is a wonderful topic. We are fortunate to have the medical treatments that are now available for HAE and more to come. I do strongly believe that we should take an integrative approach to care. Particularly for patients struggling with a condition like HAE it is important to look toward any aspect of your lives that can be ‘health promoting’. To begin our discussion, specific to HAE, we routinely discuss with patients strategies for navigating various potential ‘attack triggers’. Marc, would you like to comment on the approach you advise for patients?


Dr. R:This is a very important and difficult question. Important because it¹s clear in my clinical practice that while modern HAE medications have dramatically improved the lives of most patients, these prescriptions don’t solve every issue related to HAE. The question is difficult because very little research has been done in the area of non-pharmacologic treatment for HAE – so most of what I can say in this area is based on personal observation and speculation. The majority of our non-pharmacologic strategies involve avoiding known HAE attack triggers as a form of prevention. Triggers may include infection, trauma, medications, and stress. We try to prevent infections by recommending routine immunizations and hand-washing. Medications to avoid include estrogens and ACE-inhibitors. Trauma is a tricky one – we can plan for medical and dental procedures (though that typically involves using medication), but accidental trauma isn¹t entirely avoidable and I actually encourage my patients to ‘live their lives’ – do the things you like to do including physical activity since we can often use medications to prevent or treat any swelling that may result.


Dr. C:Thank you Marc. I would now like to turn to the role of diet. It is often said that we ‘are what we eat’. The best advice that I have heard in this regard was given by Michael Pollan: “Eat food, not too much, mostly plants”. While we are often asked by patients about possible dietary triggers or therapies I’m not aware, of any actual studies for HAE. Bruce, aren’t you planning to do a survey addressing the role of diet in HAE?


Dr. Z:Along with the entire US HAEA Medical Advisory Board, I’ll be conducting a survey of dietary history in HAE patients in which we’ll look at the relationship between diet and HAE severity. I briefly spoke about this at the most recent Patient Summit. Many of our patients and some physicians wonder if there could be a connection between diet and swelling, and we receive a surprising amount of feedback about possible relationships. To be honest, we’ve always assumed that diet is not a factor in HAE attacks, and I would guess that we’re unlikely to find any connections between diet and HAE. On the other hand, we want to look to see if there’s enough of a hint to justify a more careful study. Whether the survey is positive or negative, I plan to present the results of this survey at the next summit because I think that patients are interested in this topic.


Dr. C:Finally I’d like to turn our attention now to ‘wellness’. It will not cure HAE but the better your overall condition the better you will be able to function and be your ‘best self’. One of the themes conveyed at the Patient Summit in Denver hosted by the HAEA was to ‘get in motion’. Exercise is one of the best prescriptions we can give for any patient. We know that doing something is enormously better then doing nothing. One of our prior discussions of the QOTW (July 13, 2015) went into some detail on this topic. A life in balance is also something to strive for. Do things that are meaningful and pleasurable.

I have also been very interested in the interrelationship between stress and swelling. Anyone involved in HAE can certainly appreciate that stress is an aggravating factor for HAE, but our knowledge doesn’t extend much beyond this. We have started a study to examine this relationship—thank you to all who have participated. Hopefully we will have some answers from the study down the road as to how stress translates into swelling. For the present we can’t always alter the fact that there is stress in our lives but we can take some measures to mitigate how stress affects us. Incorporating measures involving the mind body connection is something to consider. Many of our readers don’t have the time or money for formal classes or training. Again in the ‘something is better than nothing’ category fortunately there is an app for everything. ‘Simply Being’ is an excellent mindfulness program that you can buy at the Apple Store for $1. You can set it to music or nature sounds and use it for 5 minutes. It falls into the category of something that almost anyone can do to see if it helps. Marc, what thoughts do you have for our readers on ‘wellness’ for HAE?


Dr. R:Perhaps the most intriguing potential non-pharmacologic interventions are those for mental or psychological stress. There¹s extremely little research data on these approaches, but it¹s quite clear in practice that stress exacerbates HAE in many patients so it seems reasonable that approaches to reduce or manage that stress might be beneficial. Over the years, individual patients have informed me that things such as yoga, meditation, mindfulness, exercise, and even relaxing hobbies have been helpful in reducing stress and subsequently the frequency or severity of their HAE attacks. Along these lines, an intriguing paper was recently published reporting the clinical benefit of biofeedback training in an individual with HAE (Burns, S. Appl Psychophysiol Biofeedback.40:251­256. 2015) The study subject with HAE underwent four weeks of formal biofeedback training which resulted in reductions of HAE attacks, less use of acute HAE medication, and improvement in QOL. This is a single case report, so we don¹t know how successful this approach would be for others, but it¹s an example of a type of intervention that warrants more rigorous study. Finally, studies have shown that the rates of anxiety and depression are higher in HAE than in the general population which makes sense given the severe and unpredictable nature of this chronic condition. But it’s suspected that this HAE-associated anxiety and depression may actually further fuel HAE symptoms through signals or mediators from the sympathetic nervous system. This hasn¹t all been worked out scientifically, but it could in some cases cause a ‘vicious cycle’ of mental stress triggering HAE episodes triggering more mental stress and so forth. For these reasons, I think some people benefit from counseling to approach anxiety and depression from a non-pharamcologic angle as well.


Dr C:Thank you Marc. Bruce has been very involved in helping me with the study of stress and HAE. As Marc has highlighted there had been little done to investigate this in the past. Can you elaborate on the connection of stress and swelling for our reader and some of the future non-pharmacologic pathways we are considering for intervention in the future?


Dr. Z:Let me remind our readers that HAE used to be called HANE, or Hereditary Angioneurotic Edema! This shows us that the role of stress in HAE has been appreciated for almost 130 years. Like you, I’ve seen lots of examples where stress has apparently triggered attacks. In addition, HAE itself is very stressful. I’m intrigued by the possibility of breaking this cycle in which stress provokes attacks that leads to even more stress. To really address this problem, we need to understand how stress impacts HAE. The mind and the body used to be thought of as being reasonably separate. As we’ve learned more and more about mind-body connections, it has become very clear that the mind and the immune system are constantly communicating with each other. There’s emerging evidence of both cellular and molecular links between them, many of which could potentially worsen angioedema. Our current study is looking only at the association between stress and swelling. The next phase, however, will begin to elucidate the mechanisms by which they influence each other as well as test specific interventions to improve HAE severity. We think that this will be an area where it will be possible to make large differences in HAE severity using non-pharmacologic approaches.


Dr. C:Thank you, Bruce and Marc. We hope that some of this advice is helpful to our reader and others with HAE. Remember we all need to just keep trying to do the best we can. Incorporate some of the suggestions for a healthy lifestyle if you are not already practicing them. Congratulate yourself on any positive steps that you make. Finally, to paraphrase Voltaire ‘don’t let perfect be the enemy of the good’. We look forward to our next ‘Question of the Week’.


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